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Updated: Jun 8, 2026

Protein Misfolding Cyclic Amplification of Prions
Published on: November 7, 2012
De novo prions
Abstract:
Prions are responsible for a heterogeneous group of fatal neurodegenerative diseases. They occur in three forms - sporadic, genetic, or acquired - and involve non-covalent post-translational modifications of the cellular prion protein (PrP(C)). Prions (PrP(Sc)) are characterized by their infectious properties and intrinsic ability to act as a template, converting the normal, physiological PrP(C) into the pathological form, PrP(Sc). The 'protein-only' hypothesis, postulated by Stanley B Prusiner, implies that the generation of de novo prions is possible. Exciting recent work, in vivo and in vitro, has further strengthened this postulate.
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